hrp0094p2-403 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2021

Surgical aspects of the multicentric and interdisciplinary study in minors with different sexual development

Nerea Gonzalez-Temprano , Villalón Flor , Cancela Vanesa , Nuñez Francisco Javier , Hernandez Jone Miren , Diez-Lopez Ignacio

After the evident socio-health transformation when facing intersexualities / entities with different sexual development (DSD), we wonder if clinical-care assistance has improved. Material and method Descriptive multicenter study by means of a retrospective review (2006-2019) of patients with a diagnosis of DSD in pediatric age. Interdisciplinary assessment through satisfaction surveys to families / patients.Results: 67 patients were included. 27% were op...

hrp0094p2-62 | Bone, growth plate and mineral metabolism | ESPE2021

Severe Hypophosphatemic Rickets due to Tumor-Induced Osteomalasia

Haliloglu Belma , Pehlivan Enes , Yılmaz Duygu , Turan Cift Hakan , Tasdelen Neslihan , Sav Aydın , Alan Selcuk Nalan , Turan Serap ,

Introduction: Hypophosphatemic rickets is usually due to genetic causes but in rare cases it can develop secondary to MAS, Fanconi syndrome, or oncogenic causes. Oncogenic osteomalacia, also called Tumor-Induced Osteomalacia (TIO), is a rare acquired paraneoplastic syndrome that develops as a result of excessive phosphate loss from renal tubules by FGF23 released from tumour tissue. In addition to the clinical features of rickets, gait disturbances, growth ret...

hrp0097fc6.2 | Pituitary, neuroendocrinology and puberty 1 | ESPE2023

The role of body composition and appetite-regulating hormones in idiopathic central precocious puberty and their changes during GnRH analogue therapy.

Tarçın Gürkan , Bayramoğlu Elvan , Güneş Kaya Didem , Karakaş Hasan , Turan Hande , Evliyaoğlu Olcay

Aim: This study aimed to compare the levels of appetite-regulating hormones (ghrelin, leptin, peptide-YY (PYY), neuropeptide-Y (NPY)) in girls with idiopathic central precocious puberty (ICPP) before treatment with prepubertal girls, and to evaluate changes in these hormone levels and body composition during leuprolide acetate (LA) treatment.Methods: This prospective, cross-sectional study included girls with ICPP, isola...

hrp0097fc7.1 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2023

Polygenic scores for testosterone and SHBG are associated with hormone levels in male infants

Siegfried Busch Alexander , Lindhardt Ljubicic Marie , N. Upners Emmie , B. Fischer Margit , P Hagen Casper , Juul Anders

Background: The male Hypothalamic-Pituitary-Gonadal (HPG) axis undergoes a transient activity phase during the first months of life with surging serum concentrations of reproductive hormones. Theoretically, the hormonal surge could represent a passive postnatal feedback to the withdrawal from the high sex steroid levels in pregnancy. However, we believe that minipuberty rather represents an active, tightly genetically-regulated biological process. We therefore...

hrp0097rfc7.1 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2023

Penile width increases more clearly than penile length during minipuberty: a longitudinal study of 136 healthy infant boys

Mola Gylli , Siegfried Busch Alexander , Lindhardt Ljubicic Marie , N. Upners Emmie , Bistrup Fischer Margit , Juul Anders , P. Hagen Casper

Background: In minipuberty, the male hypothalamic-pituitary-gonadal (HPG) axis is transiently activated triggering a surge in reproductive hormones causing growth of the genitalia. Longitudinal individual growth of the infant penis during minipuberty and associations to the HPG axis and IGF-I have not been thoroughly assessed.Aim: To describe the development of penile length and width as well as their relation to serum c...

hrp0097rfc7.4 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2023

Serum steroid metabolome dynamics during infancy: a prospective, longitudinal cohort of healthy boys

Siegfried Busch Alexander , Lindhardt Ljubicic Mariee , N. Upners Emmie , B. Fischer Margit , P. Hagen Casper , Frederiksen Hanne , Juul Anders

Background: The circulating steroid metabolome in boys undergoes significant changes during infancy reflecting functional and structural rearrangements of the adrenal glands as well as the transient activity of the hypothalamic–pituitary–gonadal axis, also called minipuberty. Studies investigating the serum steroid metabolome dynamics during infancy in a longitudinal manner are however sparse.Objective: We ai...

hrp0097rfc11.2 | GH and IGFs | ESPE2023

Are pappalysins and stanniocalcins involved in modifying the bioavailability of IGF-I in children with onset of type 1 diabetes mellitus?

Güemes Maria , Canelles Sandra , Martín-Rivada Álvaro , Corredor Beatriz , Barrios Vicente , Argente Jesús

Introduction: Both poor and optimal metabolic control of type 1 diabetes mellitus (T1DM) in children can impact longitudinal growth. A decrease in insulin-like growth factor (IGF)-I and its binding protein 3 (IGFBP3) has been described in these patients. New growth regulatory factors [pappalysins (PAPP-As) and stanniocalcins (STCs)] could modulate the bioavailability of IGFs by regulating the concentrations of intact and free IGFBPs.<str...

hrp0097p1-11 | Adrenals and HPA Axis | ESPE2023

Girls with Premature Adrenarche but not SGA Reach Their Target Height

Bingöl Aydın Dilek , Tin Oğuzhan , Turan Hande , Bayramoğlu Elvan , Ercan Oya , Evliyaoğlu Olcay

Introduction:Premature adrenarche (PA) is the most common cause of premature pubic and axillary hair growth before the age of 8 in girls. At the time of diagnosis, the height and bone age of the cases with premature adrenarche were determined to be higher than their peers. The long-term effects of advanced bone age on the final length of the cases are controversial. In this study, it is aimed to share the long-term follow-up data of the cases diagnosed with pr...

hrp0097p1-204 | Adrenals and HPA Axis | ESPE2023

Longitudinal Changes in Serum DLK1 Concentrations During Minipuberty in Healthy Infant Girls; Association to Changes in Linear Growth and Fat Mass

Vilmann Lea , Siegfried Busch Alexander , Lindhardt Ljubicic Marie , N. Upners Emmie , Bistrup Fischer Margit , P. Hagen Casper , Juul Anders

Background: Growth in infancy is considered primarily to be regulated by nutrition and insulin, whereas less is known about the influence of IGF-I, reproductive hormones and other factors of importance. Recently, paternally inherited genetic defects of DLK1 (Delta-like 1 homolog) were found in girls with central precocious puberty (CPP) with a metabolic phenotype. In addition, low maternal serum DLK1 concentrations were significantly lower in pregnant women wh...

hrp0097p1-419 | Bone, Growth Plate and Mineral Metabolism | ESPE2023

Evaluation of the pediatric patients diagnosed with secondary osteoporosis

Singin Berna , Donbaloğlu Zeynep , Barsal Çetiner Ebru , Aydın Behram Bilge , Ünver Tuhan Hale , Parlak Mesut

Objective: Secondary osteoporosis has a high rate of accompanying chronic diseases. We aimed to review the clinical and laboratory features, underlying causes, Dual Energy X-ray Absorptiometry (DEXA) results before and after treatment of patients diagnosed with secondary osteoporosis and to determine the relationship between this condition and fracture rates.Methods: This study was designed as a single-center, descriptiv...